Omphalocele

The term omphalocele ( from Greek ὀμφαλóς the navel and κήλη the break, also written omphalocele synonym:. Exomphalos ) is the medical term for a umbilical hernia ( umbilical hernia ): A physiological shift of some abdominal organs to the outside ( physiological umbilical hernia ) through the abdominal wall the unborn child. The error is the lack of or insufficient Back form the physiological umbilical hernia. Here, the bowel loops remain in the extraembryonic coelom and call a widening of the umbilical cord out. The Back shift should normally happen towards the end of the 3rd month of development. The defect can be detected before birth by ultrasound. The therapy consists in a surgical care.

Pathogenesis

An omphalocele occurs between day 32 and 70 of pregnancy. The regression of physiologic ( occurring in healthy individuals ) navel break in the embryonic missing, so that the midline defect results a substance, which allows the break.

Epidemiology

The incidence ( morbidity ) of omphalocele is 1:4000 - 1:5000 births. The omphalocele is not hereditary and it normally results in no increased risk for other children of the same mother. Ultimately, the cause (etiology ) of the omphalocele is currently still unclear. At about four in ten children with omphalocele are other anomalies in areas such as the heart, the kidneys, the liver and the intestine. Higher than average tendency to find Omphalozelen in children with Cantrell 's syndrome, Patau syndrome ( trisomy 13), Edwards syndrome ( trisomy 18), Fraser syndrome, Beckwith -Wiedemann syndrome, trisomy 16 and triploidy.

Diagnostics

The omphalocele can often be diagnosed prenatally by ultrasound. After birth, the malformation is obvious. Thus an earlier birth ( smaller infant ) or a cesarean section (primary cesarean section ) have to be taken to avoid or reduce the rupture risk into consideration.

Morphology

The size of the hernia sac is variable ( ping-pong ball to hand ball size). It contains an intact shell amnion, peritoneum, umbilical vessels, Wharton'sche jelly together with variable amounts of intraperitoneal organs ( intestines, liver, stomach).

Therapy

The birth should be done in a clinic with an attached children's intensive care and pediatric surgery. As a mode of birth is today elected the cesarean section ( cesarean section ) in many cases. Subsequently, the hernial sac is sterile covered. The child receives a feeding tube and is transferred to the pediatric intensive care unit for stabilization and further diagnosis.

If the umbilical cord rupture ruptured (ie the bowels are open), must be a fast operation. Otherwise, it is decided depending on the size of the sac and the surrounding circumstances, whether a primary or first operation a " hanging " of the sac is to take place. In the case of the surgery, if possible, put the intestines in the abdomen and the closed abdomen. If the abdominal cavity is too small, it will be closed by foreign material, which is then removed in one or more operations.

Course

The course is characterized by the size of the omphalocele and associated anomalies. The fare structure is often difficult, so the food must be supplied by infusion over a longer period. For larger Omphalozelen several operations until complete abdominal closure are often necessary.